[Liver and colonic manifestation of active chronic infection by Epstein Barr virus]. / Manifestación hepática y colónica de la infección crónica activa por virus de Epstein Barr.

INTRODUCTION: Chronic active Epstein Barr virus infection (CAEBV) is a rare condition, where the body is unable to counteract Epstein Barr viral replication (EBV), leading the patient to a chronic state with variable symptoms. Early recognition of infrequent or atypical clinical manifestations is relevant due to the particularities of their management and prognosis. OBJECTIVE: to describe a case of CAEBV manifes ted with colitis and hepatitis, summarizing the clinical-pathological and endoscopic characteristics and their evolution. CLINICAL CASE: A 6-year-old girl, previously healthy, presented recurrent episodes of jaundice, hepatosplenomegaly, and fever. EBV hepatitis was diagnosed with a blood viral load of 328,000 copies / mL. Her liver biopsy revealed Epstein-Barr virus-encoded small RNAs (EBER). She evolved with mucosanguineous diarrhea and weight loss; the colonoscopy showed loss of the haustral pattern, multiple aphthous ulcers covered with fibrin, and 7 million copies of EBV / gram of tissue were found in the colon. T-cell lineage infection was identified, therefore Rituximab was started, with a decrease in viral load, complete resolution of diarrhea, and improvement in liver function tests. The definitive treatment was bone marrow transplantation. CONCLUSIONS: CAEBV is a serious disor der, little documented, and should be considered in the face of a prolonged or intermittent course of hepatitis, accompanied by general and gastrointestinal manifestations such as chronic diarrhea, hematochezia, and weight loss, since its outcome without treatment can be fatal.

Nutritional benefits in pediatric patients with percutaneous endoscopic gastrostomy placement.

Gastrostomy is an enteral nutrition option. Indications for its placement are diverse, among them, the alteration in the mechanics of swallowing, frequently present in patients with neurological diseases. Nutritional recovery is the objective in these patients evaluating the nutritional status after the placement of a PEG in the pediatric population. This is a retrospective cohort conducted to evaluate nutritional recovery in pediatric patients with PEG placement. It was performed using anthropometric and biochemical parameters before and after placement. Forty-seven subjects were included, from which weight, height, and upper arm circumference were obtained, to evaluate nutritional recovery using BMI or W/L according to age. Significant nutritional improvement was demonstrated in a population of 39 patients, who had a mean follow-up of 7 months. In our population, PEG is an enteral nutrition strategy that has a significant positive impact on nutritional status in a mean of 7 months after its placement. Therefore, considering the evolution time of these patients will guide the clinician in making decisions regarding surveillance and monitoring parameters of the nutritional status.

Validez del hallazgo de nódulos en la colonoscopia para el diagnóstico de hiperplasia nodular linfoide en niños / Validity of nodules detected at colonoscopy for the diagnosis of nodular lymphoid hyperplasia in children

Resumen Introducción: la hiperplasia nodular linfoide del colon se define como > 10 nódulos linfoides visibles en colonoscopia. No existen estudios de su validez al compararlo con la histopatología. Objetivos: determinar la validez del hallazgo de nódulos en colonoscopia para el diagnóstico de hiperplasia nodular linfoide. Material y métodos: estudio prospectivo de prueba diagnóstica. Se incluyeron colonoscopias realizadas consecutivamente de 2014 al 2018 con equipos Olympus PCFQ150AI y GIFXP150N con obtención de biopsias. El criterio endoscópico fue la presencia de > 10 nódulos de 2 a 10 mm y el criterio histológico fue hiperplasia de folículos linfoides y mantos de linfocitos en lámina propia o submucosa. Los datos se analizaron en Epidat3.1. Se obtuvo la sensibilidad (S), especificidad (E), valor predictivo positivo (VPP) y negativo (VPN), y coeficientes de probabilidad positivo (LR+) y negativo (LR-) con sus intervalos de confianza. Resultados: se incluyeron 327 colonoscopias, la mediana de edad fue de 84 meses. La principal indicación para la colonoscopia fue sangrado digestivo bajo (38,8%). El hallazgo de nódulos se encontró en el 21% y el sitio de mayor frecuencia fue el colon total (46%), mientras que por histopatología se encontró hiperplasia nodular linfoide en el 38%. El hallazgo de nódulos obtuvo una S de 32% (intervalo de confianza [IC] del 95%: 24-140), E de 84% (IC 95%: 79-89), VPP de 56% (IC 95%: 44-68), VPN de 67% (IC 95%: 61-72), LR+ de 2,04 (IC 95%: 1,4-3) y LR- de 0,8 (IC 95%: 0,8-0,9). Conclusiones: la validez diagnóstica del hallazgo de nódulos en colonoscopia para hiperplasia nodular linfoide es pobre, por lo que la toma de biopsia debe recomendarse siempre.

Abstract Introduction: Nodular lymphoid hyperplasia of the colon is characterized by the presence of >10 lymphoid nodules visible in colonoscopy. There are no studies that confirm their validity when compared with histopathology. Objective: To determine the validity of nodules detected at colonoscopy for the diagnosis of nodular lymphoid hyperplasia in children. Materials and methods: Prospective study of diagnostic test accuracy. Colonoscopies performed consecutively from 2014 to 2018 using Olympus PCFQ150AI and GIFXP150N biopsy machines were included. The endoscopic criterion was the presence of >10 nodules from 2 to 10mm of diameter, while the histological criterion was presence of follicular lymphoid hyperplasia and lymphocyte mantles in lamina propia or submucosa. Data were analyzed in Epidat3.1. Sensitivity (SE), specificity (SP), positive predictive value (PPV), negative predictive value (NPV), positive likelihood ratio (LR+) and negative likelihood ratio (LR-) were obtained with their corresponding confidence intervals. Results: 327 colonoscopies were included; the median age was 84 months. The main indication for colonoscopy was lower gastrointestinal bleeding (38.8%). Nodules were found in 21% of the patients, predominantly throughout the whole colon (46%), whereas histopathology found nodular lymphoid hyperplasia in 38%. SE for the finding of nodules was 32% (95% confidence interval [CI]: 24-140), SP was 84% (95% CI: 79-89), PPV was 56% (95% CI: 44-68), NPV was 67% (95% CI: 61-72), LR+ was 2.04 (95% CI: 1.4-3) and LR- was 0.8 (95% CI: 0.8-0.9). Conclusions: The validity of the presence of nodules on colonoscopy for the diagnosis of nodular lymphoid hyperplasia is poor, so biopsy should always be performed.

Epidermolysis bullosa in children: a retrospective study in a reference hospital. / Epidermólisis ampollosa en niños: un estudio retrospectivo en un hospital de referencia.

BACKGROUND: Epidermolysis bullosa (EB) is a genodermatosis caused by mutations in the proteins of the dermal-epidermal junction, altering the epithelial cohesion, and generating blisters and shedding of skin and mucous membranes. OBJECTIVE: To describe the demographic and clinical characteristics, as well as the main complications of patients with EB attended at the National Institute of Pediatrics, in Mexico City. METHOD: An observational, descriptive, retrospective and cross-sectional study was conducted in patients under 18 years of age with diagnosis of EB. Patients with incomplete, purged or archived records were excluded. RESULTS: We included 35 patients, 17 men and 18 women with an average age of 8.94 ± 4.9 years. Patients were classified as dystrophic EB (71.4%), EB simplex (17%), junctional EB (2.9%) and Kindler syndrome (2.9%). All patients presented skin manifestations, followed by manifestations in oral mucosa (74.3%), nutritional (54.2%), gastrointestinal (51.4%), hematological (40%), ophthalmological (37.1%), musculoskeletal (34.2%) and psychosocial symptoms (34.2%). The degree of severity was variable according to the subtype; junctional EB and dystrophic EB are those that generate greater affection and comorbidity. CONCLUSIONS: EB is a serious multisystem genetic disease, which is why it requires an early diagnosis and a timely detection of complications.

INTRODUCCIÓN: La epidermólisis ampollosa (EA) es una genodermatosis causada por mutaciones en proteínas de la unión dermoepidérmica que alteran la cohesión epitelial y generan ampollas y desprendimiento de piel y mucosas. OBJETIVO: Describir las características demográficas y clínicas, así como las principales complicaciones, de los pacientes con EA atendidos en el Instituto Nacional de Pediatría, en Ciudad de México. MÉTODO: Se realizó un estudio observacional, descriptivo, retrospectivo y transversal en pacientes menores de 18 años con diagnóstico de EA. Se excluyeron los pacientes con expedientes incompletos, depurados o archivados. RESULTADOS: Se incluyeron 35 pacientes, 17 hombres y 18 mujeres, con edad media de 8.94 años (desviación estándar: 4.9), que se clasificaron como EA distrófica (71.4%), EA simple (17%), EA de unión (2.9%) o síndrome de Kindler (2.9%). Todos los pacientes presentaron manifestaciones en la piel, seguidas por manifestaciones en la mucosa oral (74.3%), nutricionales (54.2%), gastrointestinales (51.4%), hematológicas (40%), oftalmológicas (37.1%), musculoesqueléticas (34.2%) y psicosociales (34.2%). La gravedad fue variable de acuerdo con el subtipo; la EA de unión y la EA distrófica son las que generan mayor afectación y comorbilidad. CONCLUSIONES: La EA es una enfermedad genética multisistémica grave, por lo que es fundamental su diagnóstico temprano y la detección oportuna de complicaciones.

[Guía latinoamericana para el diagnóstico y tratamiento de alergia a las proteínas de la leche de vaca (GL-APLV)].

Cow's milk allergy (CMA) is an immune-based disease that has become an increasing problem. The diagnosis and management of CMA varies from one clinical setting to another and represents a challenge in pediatric practice. In addition, because nonallergic food reactions can be confused with CMA symptoms, there is an overdiagnosis of the disease. In response to these situations, pediatric specialties from recognized institutions throughout Latin America decided to develop a clinical guideline for diagnosis and management of cow's milk allergy. These guidelines include definitions, epidemiology, pathophysiology overview, clinical and evidencebased recommendations for the diagnosis and treatment of CMA. They also include prevention and prognosis sections and identify gaps in the current knowledge to be addressed through future research.